Case Reports - Two cases of primary ovarian neuroblastoma arising from mature cystic teratomas

open access article

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Highlights

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Primary ovarian neuroblastomas are extremely rare and have poor prognoses.

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This report provides results of the same combination therapy in two cases, which indicated its efficacy for ovarian neuroblasoma.(Neuroblastoma (NB) is the most common extracranial solid cancer in childhood and the most common cancer in infancy...)

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Introduction

Neuroblastoma accounts for approximately 8-10% of all tumors in children aged 15 years, and the incidence of neuroblastomas 1 in 7000 in the United States.

Neuroblastoma treatment is based on the clinical stage and categories. Surgery and/or chemotherapy are recommended. Most infants with disseminated disease have a favorable outcome after chemotherapy and surgery, while the majority of children aged > 18 months, who have advanced neuroblastoma, die of progressive disease despite intensive multimodality therapy.

Neuroblastomas develop from neural crest cells, and their anatomical locations are essentially the adrenal glands and paraspinal sites. Only a few reports of primary ovarian neuroblastomahave been reported worldwide.　Although it is commonly known that malignancy occurs in 0.3–4.8% of mature cystic teratomas of the ovary [1],neurobalstomas arising from mature cystic teratomas　are extremely rare, as we found only 4 cases published in the literature since 1982^{[2] and [5]}. Almost all ovarian neuroblastomas have very poor prognoses; however, we performed intensive treatment with a combination of surgery and chemotherapy and found no occurrence of relapse in two cases. Particularly, case 1 achieved complete remission for > 13 years in spite of multiple metastases.

Case Reports

Case1

A 22-year-old Japanese woman presented with difficulty in walking and numbness of her right thigh. It was revealed by magnetic resonance imaging (MRI) that she had aparavertebral tumor from the first to third lumbar vertebrae (Fig.1A).A computed tomography (CT) scan showed bilateral ovarian tumors (Fig.1B)...............